Background: TAKAYASU arteritis (TA) or giant cell vasculitis is the third common vasculitis after Henoch-Schoenline purpura and Kawasaki disease in children. This vasculitis usually affects large vessels and is more common in females during the childhood. Aim of this report is to present three Iranian children (two boys, one girl) with TA.Case Presentation: Mean age of our cases on admission was 10 years. Patients most commonly presented with episodes of systemic symptoms including, fever, headache, increased arterial blood pressure in one limb and convulsion. Other related symptoms were myalgia, limb pain, chest pain, and abdominal pain. Supra sternal, and abdominal bruit was noted in all patients. Angiography was performed in all cases. This revealed stenosis of the left subclavian artery and common carotid artery in one patient, generalized aortitis in one case and stenotic right renal artery in one patient. One boy was diagnosed as having systemic onset of juvenile idiopathic arthritis. One case was referred with hand pain and headache, and one case for control of hypertension. Follow-up ranged from 2 to 7 years since diagnosis. All patients were I treated with prednisone, azathioprine, aspirin, and antihypertensive drugs. Each patient received either methotrexate, or hydroxychloroquine or mycofenolate mofetil.Conclusion: Although TA is uncommon before 10th year of life, it should be considered in patients presenting with hypertension and systemic symptoms such as fever, limb pain and pulseless limb.